Wang Shuoshan, Wu Jingguo, Jing Xiaoli, Zhang Yongwei, Tang Hao, Wu Junlin
Department of General Medicine, The First Affiliated Hospital, Sun Yat Sen University, Guangzhou, People's Republic of China.
Department of Emergency, The First Affiliated Hospital, Sun Yat Sen University, Guangzhou, People's Republic of China.
Hematology. 2019 Dec;24(1):751-756. doi: 10.1080/16078454.2020.1838708.
Hemophagocytic lymphohistiocytosis (HLH) is an immune-mediated disorder caused by uncontrolled inflammatory responses and the activation of T lymphocytes. This life-threatening disease, characterized by fever, cytopenia and hepatosplenomegaly, is extremely rare during pregnancy with high mortality. Despite the improvement of treatment regimen in recent years, HLH is still a great challenge for clinicians. Here, we described a 26-year-old woman who admitted to our hospital at her first pregnancy with pyrexia. Her condition continued to deteriorate after receiving broad-spectrum antimicrobials, presenting with fever, pancytopenia, hepatosplenomegaly, ferritin ≥ 500 μg/L, hemophagocytosis and low NK-cell activity. HLH was eventually diagnosed by clinical manifestation and laboratory examination results. Then the patient recovered well after treatment with etoposide combined with ruxolitinib therapy and underwent successful induced-labor operation. Additionally, we summarized similar cases from the literature to improve the management of HLH during pregnancy. In conclusion, this study highlights the challenges and difficulties in the diagnosis and management of patients with HLH during pregnancy. Moreover, this is the first case report of etoposide combined with ruxolitinib in the treatment of patients with refractory secondary HLH during pregnancy.
噬血细胞性淋巴组织细胞增生症（HLH）是一种由不受控制的炎症反应和T淋巴细胞激活引起的免疫介导性疾病。这种危及生命的疾病，以发热、血细胞减少和肝脾肿大为特征，在妊娠期极为罕见，死亡率很高。尽管近年来治疗方案有所改进，但HLH对临床医生来说仍然是一个巨大的挑战。在此，我们描述了一名26岁的初孕女性，因发热入院。在接受广谱抗菌药物治疗后，她的病情继续恶化，出现发热、全血细胞减少、肝脾肿大、铁蛋白≥500μg/L、噬血细胞现象及自然杀伤细胞活性降低。最终通过临床表现和实验室检查结果确诊为HLH。然后该患者在接受依托泊苷联合芦可替尼治疗后恢复良好，并成功进行了引产手术。此外，我们总结了文献中的类似病例，以改善妊娠期HLH的管理。总之，本研究突出了妊娠期HLH患者诊断和管理中的挑战与困难。此外，这是首例关于依托泊苷联合芦可替尼治疗妊娠期难治性继发性HLH患者的病例报告。
